NSABP FB-10: a phase Ib/II trial evaluating ado-trastuzumab emtansine (T-DM1) with neratinib in women with metastatic HER2-positive breast cancer.

BACKGROUND: We previously reported our phase Ib trial, testing the safety, tolerability, and efficacy of T-DM1 + neratinib in HER2-positive metastatic breast cancer patients. Patients with ERBB2 amplification in ctDNA had deeper and more durable responses. This study extends these observations with in-depth analysis of molecular markers and mechanisms of resistance in additional patients. METHODS: Forty-nine HER2-positive patients (determined locally) who progressed on-treatment with trastuzumab + pertuzumab were enrolled in this phase Ib/II study. Mutations and HER2 amplifications were assessed in ctDNA before (C1D1) and on-treatment (C2D1) with the Guardant360 assay. Archived tissue (TP0) and study entry biopsies (TP1) were assayed for whole transcriptome, HER2 copy number, and mutations, with Ampli-Seq, and centrally for HER2 with CLIA assays. Patient responses were assessed with RECIST v1.1, and Molecular Response with the Guardant360 Response algorithm. RESULTS: The ORR in phase II was 7/22 (32%), which included all patients who had at least one dose of study therapy. In phase I, the ORR was 12/19 (63%), which included only patients who were considered evaluable, having received their first scan at 6 weeks. Central confirmation of HER2-positivity was found in 83% (30/36) of the TP0 samples. HER2-amplified ctDNA was found at C1D1 in 48% (20/42) of samples. Patients with ctHER2-amp versus non-amplified HER2 ctDNA determined in C1D1 ctDNA had a longer median progression-free survival (PFS): 480 days versus 60 days (P = 0.015). Molecular Response scores were significantly associated with both PFS (HR 0.28, 0.09-0.90, P = 0.033) and best response (P = 0.037). All five of the patients with ctHER2-amp at C1D1 who had undetectable ctDNA after study therapy had an objective response. Patients whose ctHER2-amp decreased on-treatment had better outcomes than patients whose ctHER2-amp remained unchanged. HER2 RNA levels show a correlation to HER2 CLIA IHC status and were significantly higher in patients with clinically documented responses compared to patients with progressive disease (P = 0.03). CONCLUSIONS: The following biomarkers were associated with better outcomes for patients treated with T-DM1 + neratinib: (1) ctHER2-amp (C1D1) or in TP1; (2) Molecular Response scores; (3) loss of detectable ctDNA; (4) RNA levels of HER2; and (5) on-treatment loss of detectable ctHER2-amp. HER2 transcriptional and IHC/FISH status identify HER2-low cases (IHC 1+ or IHC 2+ and FISH negative) in these heavily anti-HER2 treated patients. Due to the small number of patients and samples in this study, the associations we have shown are for hypothesis generation only and remain to be validated in future studies. Clinical Trials registration NCT02236000.

Reduced toxicity conditioning for hematopoietic stem cell transplantation in children with Diamond-Blackfan anemia.

Not available.

Apparent diffusion coefficient for differentiation between extra-nodal lymphoma and squamous cell carcinoma in the head and neck: a systematic review and meta-analysis.

BACKGROUND: Radiological differentiation between extra-nodal lymphoma and squamous cell carcinoma in the head and neck is often difficult due to their similarities. PURPOSE: To evaluate the diagnostic benefit of apparent diffusion coefficient (ADC) calculated from diffusion-weighted imaging (DWI) in differentiating the two. MATERIAL AND METHODS: A systematic review was performed by searching the MEDLINE, Scopus, and Embase databases in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement. Forest plots and the pooled mean difference of ADC values were calculated to describe the relationship between extra-nodal lymphoma and squamous cell carcinoma in the head and neck. Heterogeneity among studies was evaluated using the Cochrane Q test and I2 statistic. RESULTS: The review identified eight studies with 440 patients (441 lesions) eligible for meta-analysis. Among all studies, the mean ADC values of squamous cell carcinoma was 0.88 × 10-3mm2/s and that of lymphoma was 0.64 × 10-3mm2/s. In the meta-analysis, the ADC value of lymphoma was significantly lower than that of squamous cell carcinoma (pooled mean difference = 0.235, 95% confidence interval [CI] = 0.168-0.302, P <0.0001). The Cochrane Q test (chi-square = 55.7, P <0.0001) and I2 statistic (I2 = 87.4%, 95% CI = 77.4-93.0%) revealed significant heterogeneity. CONCLUSION: This study highlights the value of quantitative assessment of ADC for objective and reliable differentiation between extra-nodal lymphoma and squamous cell carcinoma in the head and neck. Conclusions should be interpreted with caution due to heterogeneity in the study data.

Comparison of Haemonetics Cell Saver 5+ and manual density separation for optimum depletion of red blood cells and preservation of CD34+ cells in major ABO-incompatible bone marrow grafts.

BACKGROUND AIMS: The current approach for preventing hemolysis of red blood cells (RBCs) in major ABO-incompatible bone marrow (BM) grafts after infusion is to deplete RBCs from BM products before transplantation. Traditionally, manual density separation (MDS) using Ficoll-Hypaque (Cytiva Sweden AB, Uppsala, Sweden has been used to accomplish RBC depletion. This process yields good CD34+ cell recovery, but it requires open manipulation and is labor-intensive and time-consuming. We hypothesized that an alternative automated method using Haemonetics Cell Saver 5+ (Haemonetics Corporation, Boston, MA, USA) would offer equivalent RBC depletion and CD34+ cell recovery. Small marrow volumes from pediatric donors can be processed using Cell Saver (CS) without adding the third-party RBCs necessary for other automated methods. METHODS: This retrospective analysis comprised data from 58 allogeneic BM grafts. RBC depletion and CD34+ cell recovery from BM using MDS (35 grafts) were compared with CS (14 grafts). Nine products underwent RBC depletion using CS with Ficoll (CS-F) when RBC volume was less than 125 mL. RESULTS: Linear regression analysis of log transformation of CD34+ cell recovery adjusted for log transformation of both baseline CD34+ cell content and baseline total volume showed no significant difference between MDS and CS (estimated coefficient, -0.121, P = 0.096). All products contained an RBC volume of less than 0.25 mL/kg post-processing. CD34+ cell recovery with CS-F was comparable to MDS and CS and suitable for pediatric recipients of allogeneic hematopoietic cell transplantation. CONCLUSIONS: We provide evidence that an automated method using Haemonetics Cell Saver 5+ achieves RBC depletion and CD34+ cell recovery comparable to MDS when adjusting for baseline factors.

Outcomes following intolerance to calcineurin inhibitor-based graft-versus-host disease prophylaxis in children after allogeneic hematopoietic cell transplantation.

Calcineurin inhibitors (CNI), cyclosporine and tacrolimus, are commonly used for pharmacologic prophylaxis of graft-versus-host disease after allogeneic hematopoietic cell transplantation (HCT). Unfortunately, their use is associated with significant toxicities. While intolerance to CNI is well defined, there is very little information on how they impact outcomes after HCT in children. Our retrospective study in a cohort of 82 children shows a high intolerance rate of 39% in this population associated with lower event-free survival and a higher transplant-related mortality.

Dural and Leptomeningeal Diseases: Anatomy, Causes, and Neuroimaging Findings.

Meningeal lesions can be caused by various conditions and pose diagnostic challenges. The authors review the anatomy of the meninges in the brain and spinal cord to provide a better understanding of the localization and extension of these diseases and summarize the clinical and imaging features of various conditions that cause dural and/or leptomeningeal enhancing lesions. These conditions include infectious meningitis (bacterial, tuberculous, viral, and fungal), autoimmune diseases (vasculitis, connective tissue diseases, autoimmune meningoencephalitis, Vogt-Koyanagi-Harada disease, neuro-Behçet syndrome, Susac syndrome, and sarcoidosis), primary and secondary tumors (meningioma, diffuse leptomeningeal glioneuronal tumor, melanocytic tumors, and lymphoma), tumorlike diseases (histiocytosis and immunoglobulin G4-related diseases), medication-induced diseases (immune-related adverse effects and posterior reversible encephalopathy syndrome), and other conditions (spontaneous intracranial hypotension, amyloidosis, and moyamoya disease). Although meningeal lesions may manifest with nonspecific imaging findings, correct diagnosis is important because the treatment strategy varies among these diseases. ©RSNA, 2023 Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. Quiz questions for this article are available through the Online Learning Center.

Performance of axial diameter on MR imaging for identification of retropharyngeal lymph node metastases in head and neck cancer: a systematic review and meta-analysis.

PURPOSE: To summarize previous studies' data and to calculate the diagnostic performance of minimum axial diameter (MIAD) and maximum axial diameter (MAAD) on each of the cutoff values in retropharyngeal lymph node (RPLNs) metastases in head and neck cancer. METHODS: MEDLINE, Scopus, and Embase databases were searched for systematic review. Meta-analysis was performed to summarize estimates of sensitivity, specificity, and diagnostic odds ratio (DOR) and generate summary recipient operator characteristic (sROC). RESULTS: The review identified 5 studies with a total of 634 patients (971 lesions) that were eligible for the meta-analysis. The estimated sensitivity, specificity, and DOR at MIAD 5 mm cutoff and MIAD 6 mm cutoff were 89.8% and 74.3%, 82.7% and 92.7%, and 39.1 and 57.9, respectively. The estimated sensitivity, specificity, and DOR at MAAD 7 mm cutoff and MAAD 8 mm cutoff were 90.3% and 84.7%, 62.7% and 79.9%, and 17.8 and 21.7, respectively. The AUCs of sROC at MIAD 5 mm cutoff and MIAD 6 mm cutoff were 0.922 and 0.943. At MAAD 7 mm and MAAD 8 mm, they were 0.840 and 0.888. CONCLUSION: The diagnostic performance of the MIAD 6 mm cutoff in RPLN metastases from head and neck cancer was 2% higher than the MIAD 5 mm cutoff. The diagnostic performance of MIAD was higher than that of MAAD.

Precise differentiation between jugular foramen paragangliomas and metastases: utility of diffusion-weighted and dynamic contrast-enhanced magnetic resonance imaging.

PURPOSE: This study tested the utility of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced imaging (DCE-MRI) in differentiating paragangliomas and metastases in the jugular foramen in combination with conventional imaging. METHODS: Forty-nine consecutive patients with paragangliomas or metastases between January 2015 and April 2022 were included in this retrospective study. All patients had pretreatment DWI and DCE-MRI. Between paragangliomas and metastases, normalized apparent diffusion coefficient (nADCmean) and DCE-MRI parameters were compared along with conventional imaging features (enhancement pattern, presence of flow voids, cystic/necrotic change, and bone erosion). The diagnostic performance was tested using receiver operating characteristic (ROC) analysis. RESULTS: Thirty-five paragangliomas (5 male; median 49 years) and 14 metastases (9 male; median 61 years) were analyzed. The most common 3 primary cancers included 4 lung cancers, 3 breast cancers, and 3 melanomas. The presence of flow void was significantly different between paragangliomas and metastases (21/35 vs 2/14; P = 0.0047) in conventional imaging features, while fractional plasma volume (Vp) was significantly different between the two tumor types (median 0.46 vs 0.19; P < 0.001) in DWI and DCE-MRI parameters. The areas under the ROC curves (AUCs) of the presence of flow void and Vp were 0.72 and 0.93, respectively. The AUC of the combination of the presence of flow void and Vp was 0.95 and significantly improved compared to that of the presence of flow void (P < 0.001). CONCLUSION: Adding DCE-MRI to the head and neck protocol can aid in the precise differentiation between jugular foramen paragangliomas and metastases.

Dynamic contrast-enhanced MRI parameters and apparent diffusion coefficient as treatment response markers of skull base osteomyelitis: a preliminary study.

Purpose: Currently, there is no definitive consensus on the optimal imaging modality for determining the treatment response in patients with skull base osteomyelitis (SBO). This study aimed to investigate the utility of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) parameters and apparent diffusion coefficient (ADC) as treatment response markers of SBO. Material and methods: This study included 6 patients with SBO, who underwent both pre- and post-treatment DCE-MRI and diffusion-weighted imaging (DWI). Quantitative DCE-MRI parameters and ADC of the region-of-interest were analysed. These normalized parameters were calculated by dividing the region-of-interest by the reference region. The Wilcoxon signed rank test was used to compare these parameters between pre- and post-treatment periods. Results: The normalized fraction of the extravascular extracellular space (Ve) and ADC of the post-treatment status of SBO was significantly lower than those of pre-treatment measures (p = 0.03). The normalized fraction of blood plasma (Vp), normalized rate of transfer from the blood plasma into the extravascular extracellular space (Ktrans), and normalized backflow leakage of material from the extravascular extracellular space into the blood plasma (Kep) demonstrated no significant differences between pre- and post-treatment. Conclusions: DCE-MRI parameters Ve and ADC demonstrated a significant reduction when comparing measures across the pre- and post-treatment periods. These parameters may potentially serve as a valuable surrogate treatment response marker for SBO activity.

Dynamic susceptibility contrast-MRI parameters, ADC values, and the T2-FLAIR mismatch sign are useful to differentiate between H3-mutant and H3-wild-type high-grade midline glioma.

OBJECTIVES: Diffuse midline gliomas, H3K27-altered (DMG-A), are malignant gliomas with an unfavorable prognosis. Knowledge of dynamic susceptibility contrast (DSC) MRI findings and imaging differences with high-grade midline glioma without H3K27 alteration (DMG-W) has been limited. We compared the DSC, ADC, and conventional MRI findings between DMG-A and DMG-W. METHODS: In this single institutional retrospective study, the electronic database of our hospital between June 2015 and May 2021 was searched. Twenty and 17 patients with DMG-A (median, 13 years; range, 3-52 years; 11 females) and DMG-W (median, 40 years; 7-73 years; 9 females), respectively, were found. Normalized relative cerebral blood flow (nrCBF) and normalized corrected relative cerebral blood volume (ncrCBV); normalized maximum, mean, and minimum ADC values; and the prevalence of T2-FLAIR mismatch sign were compared between the two groups using Mann-Whitney U tests and Fisher's exact test. RESULTS: The nrCBF and ncrCBV were significantly lower in DMG-A compared with DMG-W (nrCBF: median 0.88 [range, 0.19-2.67] vs. 1.47 [range, 0.57-4.90] (p < 0.001); ncrCBV: 1.17 [0.20-2.67] vs. 1.56 [0.60-4.03] (p = 0.008)). Normalized maximum ADC (nADCmax) was significantly higher in DMG-A (median 2.37 [1.25-3.98] vs. 1.95 [1.23-2.77], p = 0.02). T2-FLAIR mismatch sign was significantly more common in DMG-A (11/20 (55.0%) vs. 1/17 (5.9%), p = 0.0017). When at least two of nrCBF < 1.11, nADCmax ≥ 2.48, and T2-FLAIR mismatch sign were positive, the diagnostic performance was the highest with accuracy of 0.81. CONCLUSION: DSC-MRI parameters, ADC values, and the T2-FLAIR mismatch sign are useful to differentiate between DMG-A and DMG-W. KEY POINTS: • Diffuse midline glioma, H3K27-altered (DMG-A), showed a significantly lower normalized relative cerebral blood flow and volume compared with H3K27-wild-type counterparts (DMG-W). • T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign was significantly more frequent in DMG-A compared to DMG-W. • Indicators that combined DSC parameters, ADC values, and T2-FLAIR mismatch sign, with or without age, are useful to distinguish the two tumors.

Preoperative prediction for mastoid extension of middle ear cholesteatoma using temporal subtraction serial HRCT studies.

OBJECTIVES: This study investigated the utility of temporal subtraction computed tomography (TSCT) obtained with temporal bone high-resolution computed tomography (HRCT) for the preoperative prediction of mastoid extension of middle ear cholesteatomas. METHODS: Twenty-eight consecutive patients with surgically proven middle ear cholesteatomas were retrospectively evaluated. The presence of black color in the mastoid region on TSCT suggested progressive changes caused by bone erosion. Enlarged width of the anterior part of mastoid on HRCT was interpreted as suggestive of mastoid extension. Fisher's exact test was used to compare the widths and black color on TSCT for cases with and without mastoid extension. The diagnostic accuracy of TSCT and HRCT for detecting mastoid extension and interobserver agreement during the evaluation of black color on TSCT were calculated. RESULTS: There were 15 cases of surgically proven mastoid extension and 13 cases without mastoid extension. Patients with black color on TSCT were significantly more likely to have a mastoid extension (p < 0.001). The sensitivity and specificity of TSCT were 0.93 and 1.00, respectively. Patients in whom the width of the anterior part of the mastoid was enlarged were significantly more likely to have a mastoid extension (p = 0.007). The sensitivity and specificity of HRCT to detect the width of the anterior part of the mastoid were 0.80 and 0.77, respectively. Interobserver agreement during the evaluation of TSCT findings was good (k = 0.71). CONCLUSIONS: This novel TSCT technique and preoperative evaluations are useful for assessing mastoid extension of middle ear cholesteatomas and making treatment decisions. KEY POINTS: •TSCT shows a clear black color in the mastoid region when the middle ear cholesteatoma is accompanied by mastoid extension. •TSCT obtained with preoperative serial HRCT of the temporal bone is useful for assessing mastoid extension of middle ear cholesteatomas.

NSABP FC-6: Surgical conversion rate in colorectal cancer patients with unresectable, KRAS wild-type liver metastases receiving mFOLFOX7 plus cetuximab.

PURPOSE: This study sought to determine the R0 resection rate in KRAS wild-type (WT), liver-only metastatic colorectal cancer (CRC) patients initially identified as having unresectable disease who were treated with FOLFOX7 plus cetuximab. Exploratory molecular analyses were undertaken before and after treatment. METHODS: Twenty patients were enrolled. None had prior adjuvant chemotherapy. Cetuximab was added to a FOLFOX7 backbone and administered at 500 mg/m2 every 14 days with dose reductions to 400 and 300 mg/m2 in the event of toxicity. In the absence of toxicity, dose-escalations to 600, 700, and 800 mg/m2 were allowed. The mean dose of cetuximab (mg/m2 /week) throughout the study was 289 mg/m2 . Paired samples were collected for correlative studies, where feasible. RESULTS: We assessed the conversion rates from unresectable to resectable in hepatic-only, KRAS exon 2 WT mCRC. Seventeen of 20 patients undergoing chemotherapy were considered resectable by imaging criteria; R0 resection was achieved in 15/20 patients. Molecular profiling revealed heterogeneity between patients at the gene-expression, pathway signaling, and immune-profile levels. CONCLUSIONS: Although 15/20 (75%) converted to R0 resection, by 2 years, 10/15 R0 resections had recurred. Therefore, chemotherapy plus cetuximab is of limited long-term benefit in this setting. ctDNA analysis may guide additional therapy including immunotherapy.

Outcomes of patients who underwent treatment for anti-HLA donor-specific antibodies before receiving a haploidentical hematopoietic cell transplant.

Pediatric and adolescent and young adult (AYA) patients who receive many blood product transfusions, such as individuals with sickle cell disease (SCD), severe aplastic anemia (SAA) or indolent hematologic malignancies, are at high risk for developing donor-specific antibodies (DSA). DSAs with mean fluorescence intensity (MFI) greater than 5000 have been associated with significant graft failure, but lower MFI values between 2000 and 5000 may result in poor graft function after hematopoietic cell transplant (HCT). Desensitization strategies have been developed to reduce the DSA burden in HCT recipients before graft infusion, but the experience with these strategies in the pediatric and AYA populations is not well described in the literature. Here, we describe our experience with successful desensitization by using a combination of treatment strategies in five pediatric and AYA patients, including a novel use of daratumumab in a young adult patient who had refractory DSAs and had suffered serious side effects from conventional desensitization strategies. The presence of elevated DSAs in pediatric and AYA recipients of a human leukocyte antigen (HLA)-mismatched haploidentical HCT can be overcome by a multipronged treatment strategy.

Major Changes in 2021 World Health Organization Classification of Central Nervous System Tumors.

The World Health Organization (WHO) published the fifth edition of the WHO Classification of Tumors of the Central Nervous System (WHO CNS5) in 2021, as an update of the WHO central nervous system (CNS) classification system published in 2016. WHO CNS5 was drafted on the basis of recommendations from the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) and expounds the classification scheme of the previous edition, which emphasized the importance of genetic and molecular changes in the characteristics of CNS tumors. Multiple newly recognized tumor types, including those for which there is limited knowledge regarding neuroimaging features, are detailed in WHO CNS5. The authors describe the major changes introduced in WHO CNS5, including revisions to tumor nomenclature. For example, WHO grade IV tumors in the fourth edition are equivalent to CNS WHO grade 4 tumors in the fifth edition, and diffuse midline glioma, H3 K27M-mutant, is equivalent to midline glioma, H3 K27-altered. With regard to tumor typing, isocitrate dehydrogenase (IDH)-mutant glioblastoma has been modified to IDH-mutant astrocytoma. In tumor grading, IDH-mutant astrocytomas are now graded according to the presence or absence of homozygous CDKN2A/B deletion. Moreover, the molecular mechanisms of tumorigenesis, as well as the clinical characteristics and imaging features of the tumor types newly recognized in WHO CNS5, are summarized. Given that WHO CNS5 has become the foundation for daily practice, radiologists need to be familiar with this new edition of the WHO CNS tumor classification system. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. ©RSNA, 2022.

Deadly Fungi: Invasive Fungal Rhinosinusitis in the Head and Neck.

Invasive fungal rhinosinusitis (IFRS) is a serious infection that is associated with high morbidity and mortality rates. The incidence of IFRS has been increasing, mainly because of the increased use of antibiotics and immunosuppressive drugs. Rhino-orbital cerebral mucormycosis has recently reemerged among patients affected by COVID-19 and has become a global concern. The detection of extrasinus involvement in its early stage contributes to improved outcomes; therefore, imaging studies are essential in establishing the degree of involvement and managing the treatment properly, especially in immunocompromised patients. The common sites of extrasinus fungal invasion are the intraorbital, cavernous sinus, and intracranial regions. Fungi spread directly to these regions along the blood vessels or nerves, causing devastating complications such as optic nerve ischemia or compression, optic neuritis or perineuritis, orbital cellulitis, cavernous sinus thrombosis, mycotic aneurysm, vasculitis, internal carotid arterial occlusion, cerebral infarction, cerebritis, and brain abscess. IFRS has a broad imaging spectrum, and familiarity with intra- and extrasinonasal imaging features, such as loss of contrast enhancement of the affected region, which indicates tissue ischemia due to angioinvasion of fungi, and the surrounding anatomy is essential for prompt diagnosis and management. The authors summarize the epidemiology, etiology, risk factors, and complications of IFRS and review the anatomy and key diagnostic imaging features of IFRS beyond the sinonasal regions. ©RSNA, 2022.

Central Nervous System Systemic Lupus Erythematosus: Pathophysiologic, Clinical, and Imaging Features.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiple immunologic abnormalities and has the potential to involve the central nervous system (CNS). The prevalence of SLE seems to be growing, possibly because of earlier diagnosis and improved survival; however, the associated mortality is still high. The mortality is associated with disease-related risk factors such as lupus disease activity, young age, and organ damage or with antiphospholipid syndrome (APS). Neuropsychiatric SLE (NPSLE), which is caused by SLE-related CNS involvement, comprises a broad range of neurologic and psychiatric manifestations with varying severity, which can make this disease indistinguishable from other conditions that are unrelated to SLE. No unifying pathophysiology has been found in the etiology of NPSLE, suggesting that this condition has multiple contributors such as various immune effectors and the brain-intrinsic neuroimmune interfaces that are breached by the immune effectors. The postulated neuroimmune interfaces include the blood-brain barrier, blood-cerebrospinal fluid barrier, meningeal barrier, and glymphatic system. On the basis of the immunologic, pathologic, and imaging features of NPSLE, the underlying pathophysiology can be classified as vasculitis and vasculopathy, APS, demyelinating syndrome, or autoimmune antibody-mediated encephalitis. Each pathophysiology has different imaging characteristics, although the imaging and pathophysiologic features may overlap. Moreover, there are complications due to the immunocompromised status caused by SLE per se or by SLE treatment. Radiologists and clinicians should become familiar with the underlying mechanisms, radiologic findings, and complications of NPSLE, as this information may aid in the diagnosis and treatment of NPSLE. Online supplemental material is available for this article. ©RSNA, 2022.

Comprehensive radiological features of laryngeal sarcoidosis: cases series and systematic review.

PURPOSE: To comprehensively summarize the characteristic radiological findings of laryngeal sarcoidosis. METHODS: We reviewed patients with laryngeal sarcoidosis who underwent computed tomography (CT) and/or magnetic resonance imaging (MRI) and included 8 cases from 8 publications that were found through a systematic review and 6 cases from our institutions. Two board-certified radiologists reviewed and evaluated the radiological images. RESULTS: Almost all cases exhibited supraglottic lesions 13/14 (92.9%) and most of them involved aryepiglottic folds (12/13, 92.3%), epiglottis (11/14, 78.6%), and arytenoid region (10/14, 71.4%). Most lesions were bilateral (12/14, 85.7%). All cases showed well-defined margins and a diffuse swelling appearance (14/14, 100%). Non-contrast CT revealed a low density (4/5, 80%). The contrast-enhanced CT showed a slight patchy enhancement predominantly at the margin of the lesion in most cases (12/13, 92.3%). In one case, T2-weighted images showed high signal intensity peripherally and low signal intensity centrally (1/1, 100%). Gadolinium-enhanced MRI showed moderate heterogeneous enhancement predominantly at the margin of the lesion (2/2, 100%). In one case, diffusion-weighted imaging showed intermediate signal intensity; the apparent diffusion coefficient value was 2.4 × 10-3 mm2/s. The larynx was the only region affected by sarcoidosis in 57.1% (8/14) of the cases. Involvement of the neck lymph nodes and distant organs was observed in 4/14 (28.6%) patients, respectively. CONCLUSION: We summarized the CT and MRI findings of patients with laryngeal sarcoidosis. Knowledge of these characteristics is expected to facilitate prompt diagnosis and appropriate management.

Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature.

PURPOSE: Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) is a newly recognized brain tumor with genetic abnormalities frequently involving either BRAF or FGFR2/FGFR3. There are few publications available about the neuroradiological features of PLNTY. In this systematic review, we assessed the demographic, clinical, and neuroradiological features of PLNTY. METHODS: Literature data were extracted from database searches in MEDLINE and SCOPUS databases up to June 10, 2021. Studies reporting on pathologically proven PLNTY with neuroradiological findings were included. After reviewing 103 abstracts, 9 articles encompassing 19 cases met the inclusion criteria. We also added five patients from our hospital. The correlations between the presence of "transmantle-like sign" and the following three factors: duration of seizures; tumor size; and pathologically proven cortical dysplasia, were examined. RESULTS: The median patient age was 15.5 years (range, 5-57 years), and 15/24 (62.5%) were female. All tumors were localized supratentorialy. The main radiological features included cortical or subcortical masses (95.8%) in the temporal lobe (66.7%), calcification (83.3%), well-defined margins (72.7%), solid and cystic components (66.6%), and T2-weighted imaging (T2WI) hyperintensity (50.0%). The duration of seizure was significantly longer (positive vs. negative (median [range]), 24 months [6 - 96 months] vs. 5 months [1 - 12 months], p = 0.042), and the presence of the cortical dysplasia was significantly more frequent (3/8 vs 0/16, p = 0.042) in the patients with transmantle-like sign. CONCLUSION: PLNTY typically represents a calcified, well-defined mass in the supratentorial cortical or subcortical regions. The radiological findings defined here could facilitate the diagnosis of PLNTY.

Radiological features of human papillomavirus-related multiphenotypic sinonasal carcinoma: systematic review and case series.

PURPOSE: To comprehensively summarize the radiological characteristics of human papillomavirus (HPV)-related multiphenotypic sinonasal carcinomas (HMSCs). METHODS: We reviewed the findings for patients with HMSCs who underwent computed tomography (CT) and/or magnetic resonance imaging (MRI) and included nine cases from nine publications that were identified through a systematic review and three cases from our institution. Two board-certified radiologists reviewed and evaluated the radiological images. RESULTS: The locations in almost all cases included the nasal cavity (11/12, 91.7%). The involved paranasal sinuses included the ethmoid sinus (6/12, 50.0%) and maxillary sinus (3/12, 25.0%). The mean long diameter of the tumors was 46.3 mm. The margins in 91.7% (11/12) of the cases were well-defined and smooth. Heterogeneous enhancement on contrast-enhanced CT, heterogeneous high signal intensities on T2-weighted images and heterogeneous enhancement on gadolinium-enhanced T1-weighted images were noted in 2/2, 5/5, and 8/8 cases, respectively. Mean apparent diffusion coefficient values in two cases of our institution were 1.17 and 1.09 × 10-3 mm2/s. Compressive changes in the surrounding structures were common (75%, 9/12). Few cases showed intraorbital or intracranial extension. None of the cases showed a perineural spread, neck lymph node metastasis, or remote lesions. CONCLUSIONS: We summarized the CT and MRI findings of HMSCs. Knowledge of such characteristics is expected to facilitate prompt diagnosis and appropriate management.

Improving MR Image Quality in Patients with Metallic Implants.

The number of implanted devices such as orthopedic hardware and cardiac implantable devices continues to increase with an increase in the age of the patient population, as well as an increase in the number of indications for specific devices. Many patients with these devices have or will develop clinical conditions that are best depicted at MRI. However, implanted devices containing paramagnetic or ferromagnetic substances can cause significant artifact, which could limit the diagnostic capability of this modality. Performing imaging with MRI when an implant is present may be challenging, and there are numerous techniques the radiologist and technologist can use to help minimize artifacts related to implants. First, knowledge of the presence of an implant before patient arrival is critical to ensure safety of the patient when the device is subjected to a strong magnetic field. Once safety is ensured, the examination should be performed with the MRI system that is expected to provide the best image quality. The selection of the MRI system includes multiple considerations such as the effects of field strength and availability of specific sequences, which can reduce metal artifact. Appropriate patient positioning, attention to MRI parameters (including bandwidth, voxel size, and echo), and appropriate selection of sequences (those with less metal artifact and advanced metal reduction sequences) are critical to improve image quality. Patients with implants can be successfully imaged with MRI with appropriate planning and understanding of how to minimize artifacts. This improves image quality and the diagnostic confidence of the radiologist. ©RSNA, 2021.